Occupation-based hand therapy in a baker with monomelic amyotrophy (distal segmental spinal muscular atrophy or Hirayama disease)

Abstract

The aim of this case report is twofold: the very rare Hirayama disease is discussed, including the impact on the patient’s functioning. The unique way to solve an orthesis problem is described, by observing patient’s performance meticulously. Various hand function assessments were used to cover every level of the International Classification of Functioning model. An occupation-based approach was followed by performing a working visit during which the essence of his treatment problem became apparent. The development of a new adjustable brace is described. Combining two sorts of wrist positions in one splint is a novel approach that could be helpful for patients with other conditions. A work visit was helpful to focus on the participation level. The occupation-based intervention resulted in the patient’s ability to resume work.